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Atypical presentation of IgG4 sclerosing cholangitis
Harrison Roocroft1, Prashantha Sambaiah1, Ahmed Monier1
1Gastroenterology, James Cook University Hospital, Middlesbrough, UK.
BMJ Case Reports
|November 19, 2021
Summary
IgG4-related sclerosing cholangitis (ISC) is typically seen in older adults. This report details a rare case of ISC and autoimmune pancreatitis in a 20-year-old, highlighting atypical presentation and steroid treatment.
Area of Science:
- Gastroenterology and Hepatology
- Immunology
- Rare Diseases
Background:
- IgG4-related sclerosing cholangitis (ISC) is a rare fibroinflammatory condition.
- ISC commonly affects individuals over 60, often presenting with obstructive jaundice.
- Association with autoimmune pancreatitis is frequent in ISC cases.
Purpose of the Study:
- To report a unique case of IgG4-related sclerosing cholangitis (ISC) in a young patient.
- To describe the diagnostic process and management of this rare condition in an atypical demographic.
- To emphasize the importance of considering ISC in younger patients with relevant symptoms.
Main Methods:
- Case report of a 20-year-old male patient.
- Diagnostic workup included magnetic resonance cholangiopancreatography (MRCP).
- Clinical presentation and diagnostic findings were analyzed.
Main Results:
- The patient presented with acute abdominal pain, an unusual initial symptom for ISC.
- Diagnosis confirmed co-existing IgG4-related sclerosing cholangitis (ISC) and autoimmune pancreatitis.
- Magnetic resonance cholangiopancreatography (MRCP) was crucial for diagnosis.
Conclusions:
- IgG4-related sclerosing cholangitis (ISC) can occur in young adults, challenging typical age-related presentations.
- Early diagnosis and prompt steroid treatment are vital for managing ISC and associated autoimmune pancreatitis.
- This case underscores the need for broader differential diagnoses in young patients with obstructive jaundice symptoms.
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